Antiphospholipid Syndrome Page 1

Antiphospholipid Syndrome
Antiphospholipid antibody syndrome (commonly called antiphospholipid syndrome or APS) is a recently
identified autoimmune disease present mostly in young women. Those with APS make abnormal
proteins called antiphospholipid autoantibodies in the blood. This causes blood to flow improperly and
can lead to dangerous clotting in arteries and veins, problems for a developing fetus and pregnancy
miscarriage. People with this disorder may otherwise be healthy, or they also may suffer from an
underlying disease, most frequently systemic lupus erythematosus (commonly called SLE).
Fast facts
Antiphospholipid antibodies are present in 15-20% of all cases of deep vein thrombosis (blood
clots) and in one-third of new strokes occurring in people under the age of 50.
Antiphospholipid antibodies are a major cause of recurrent miscarriages and pregnancy
complications when no other causes are found.
Once the disease is diagnosed, adequate therapy in most cases can prevent the recurrence of
the symptoms.

What is APS?
APS is an autoimmune disease which can cause frequent clotting in arteries and veins and/or
miscarriages. The clotting results from the presence of proteins in the blood called anti-phospholipid
autoantibodies (commonly called aPL) formed against the persons own tissues. These autoantibodies
interfere with coagulation, leading to increased clot formation or thrombosis (in which blood flow stops
due to a clot).

The damage caused by this clotting can vary depending on the site of the clot. For instance, repeated
small clots in the heart can cause heart valve thickening or damage, with the risk of releasing clots into
blood (called an arterial embolism). aPL also may be associated with heart attacks in young people
without any known cardiac risk factors. Blood clots in the arteries in the heart can lead to heart attacks,

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while blood clots in the arteries in the brain can result in strokes. Blood clots from aPL can occur
anywhere in the circulation and can affect any organ in the body.
Clots forming in the veins most frequently occur in the lower legs. Blood clots in the leg veins can break
off and travel to the lung, causing a very serious condition called pulmonary embolism. Pulmonary
embolism blocks blood flow to the lung and decreases the amount of oxygen in the blood.
In a few cases, repeated thrombotic events may take place in a short time, leading to the progressive
damage of several organs. This acute and life-threatening condition is called catastrophic APS.
Patients with APS may suffer from other problems including low number of platelets, mottled purplish
discoloration of the skin (livedo reticularis), and skin ulcerations.
For pregnant women, aPL can lead to early and late miscarriage, and pre-eclampsia (high blood pressure
and protein in the urine during pregnancy). Originally it was suggested that aPL were responsible for
clots in the placentas blood vessels, causing fetal growth retardation. aPL also may directly attack the
placental tissues, blocking their growth and development.
What causes APS?
Why patients develop these autoantibodies is not completely understood. The production of these
autoantibodies likely is triggered by an environmental factor, such as an infection occurring in an
individual with a genetic background that makes him or her more susceptible to the disease.

aPL can be present in the bloodstream for a long time, but
thrombotic events result only occasionally. aPL increases
the risk for blood clotting, but thrombosis usually occurs
when other conditions that favor clotting are present, such
as prolonged inactivity(e.g., being restricted to bed),
surgery or pregnancy. Additional risk factors for thrombosis
are hypertension, obesity, smoking, atherosclerosis
(hardening of the arteries), and use of estrogens (birth
control pills).
Who gets APS?
APS affects women five times more commonly than men. It
is typically diagnosed between the ages of 30 and 40. While
up to 40% of patients with SLE will test positive for the anti-
phospholipid autoantibodies, only half will develop
thrombosis and/or experience miscarriages.
Like most autoimmune disorders, APS has a genetic component, although there is not a direct
transmission from parent to offspring.



For pregnant women, aPL can lead to
early and late miscarriage, and pre-
eclampsia (high blood pressure and
protein in the urine during
pregnancy).


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How is APS diagnosed?
The diagnosis of APS is made by testing the blood of patients with thrombosis and/or recurrent
miscarriages for aPL. Screening is done using two kinds of tests.
Tests may vary because of the differences in the aPL from patient to patient. Each single test cannot
detect all of the possible autoantibodies, so their combined use is strongly advised. At least one of these
tests must prove positive, and be confirmed on two occasions no less than three months apart. In
general, the higher the level of the test and the greater number of positive tests increases the risk of
developing symptoms. Having positive blood tests alone in the absence of a clot does not make a
diagnosis of aPL. There are healthy people who carry these clotting proteins in their blood who never
have a clot in their lifetimes.
How is APS treated?
Most often, aPL is detected after a thrombotic (clotting) event or recurrent miscarriages. Therefore, the
main goal of therapy is prevention of recurrences, given that the presence of the antibodies puts the
patient at strong risk for future episodes.

Vascular events. Acute thrombotic events are treated with
anticoagulants (blood thinners), initially with intravenous
heparin and then followed by oral warfarin (Coumadin). In
serious situations, some patients also are given compounds
that dissolve clots quickly.
In patients with aPL, oral anticoagulation is required to avoid
recurrences of venous blood clots, possibly over a period of
years. For arterial events, recurrences also are prevented with
drugs that inhibit platelets, such as aspirin and clopidogrel
(Plavix).
Obstetrical manifestations. Subcutaneous injections of heparin
and low-dose aspirin are the standard therapy for preventing
miscarriages. The therapy is started at the beginning of the
pregnancy and continued in the period immediately after the
delivery. This therapeutic approach has been shown to be
effective in the majority of the cases, with delivery of healthy
babies. In non-responsive cases, alternative therapies such as
intravenous immunoglobulin infusions and administration of
corticosteroids (prednisone) may help.
Pregnant women who had previous blood clots may receive the
same combination of heparin and low-dose aspirinbut with higher doses of heparinbecause of the
increased risk of blood clots.
The therapy with heparin and aspirin has been shown to be safe for both the mother and the baby.

Subcutaneous injections of
heparin and low-dose aspirin are
the standard therapy for
preventing new miscarriages.


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When antibodies are detected in patients with no prior thrombotic events or miscarriages, the need for
preventive therapy must be evaluated case by case. However, it is generally accepted that treatment is
not necessary if no additional risk factors for clotting are present.
Living with APS
The need for a long-term oral anticoagulant (blood thinning) therapy significantly affects the lifestyle of
the patients, creating the need for regular monitoring of the anticoagulation (blood-thinning) effect and
special attention paid to the diet and to situations with a bleeding risk (e.g., falls). Correction of
conventional risk factors for thrombosis (diabetes, high blood pressure, high cholesterol, obesity, and
smoking) is mandatory in APS patients. Estrogen therapy for birth control or menopausal symptoms
should be avoided.
Present treatment for the prevention of the obstetrical manifestations is quite effective. The majority of
the women can have healthy babies.
While APS is an autoimmune disease, its diagnosis does not mean that a patient will develop another
autoimmune condition.
Points to remember
The presence of aPL represents an important risk factor for recurrent thrombosis and
miscarriages.
Diagnosis and treatment is very important.
The mainstay of treatment is the prevention of blood clots through oral anticoagulation (blood
thinning) or anti-platelet drugs.
Risk factors for thrombosis must be addressed. These include diabetes; hypertension or high
blood pressure; hypercholesterolemia or high cholesterol; obesity; smoking; and estrogen
therapy for menopause or contraception.
To find a rheumatologist
For a listing of rheumatologists in your area, click here. Learn more about rheumatologists and
rheumatology health professionals.

For more information
If you want more information on this or any other form of arthritis, contact the Arthritis Foundation at
(800)283-7800 or visit the Arthritis Foundation Web site at www.arthritis.org.

Updated March 2013
Written by Pier Luigi Meroni, MD, and reviewed by the American College of Rheumatology Communications and
Marketing Committee.
This patient fact sheet is provided for general education only. Individuals should consult a qualified health care
provider for professional medical advice, diagnosis and treatment of a medical or health condition.
2013 American College of Rheumatology